Abstract:

Recently, there has been some new research progresses about pathogenic genes of hereditary tubule-interstitial nephritis, which has been confirmed as 20 kinds of pathogenic genes of nephronophthisis. Kidney Disease: Improving Global Outcomes (KDIGO) proposes adoption of a new terminology “Autosomal Dominant Tubulointerstitial Kidney Disease (ADTKD)” and suggests diagnostic criteria for autosomal dominant tubuleinterstitial kidney disease. There has been confirmed as 4 kinds of pathogenic genes of ADTKD (MUC1, UMOD, REN, HNF1B). The study of pathogenic genes, pathologic changes and clinical symptoms has great significance for the prevention, gene diagnosis, treatment and prognosis of hereditary tubule-interstitial nephritis.